What are the guidelines for using Hemarate FA (Antihemophilic Factor VIII) in pregnant women with Hemophilia A?

Guidelines for Using Hemarate FA (Antihemophilic Factor VIII) in Pregnant Women with Hemophilia A

For pregnant women with Hemophilia A, low-molecular-weight heparin (LMWH) is recommended over unfractionated heparin (UFH) for the prevention and treatment of venous thromboembolism (VTE). 1

Understanding Hemophilia A in Pregnancy

Hemophilia A is an X-linked bleeding disorder that is rare in females. When it does occur in women, pregnancy presents unique challenges that require specialized management:

• Pregnancy creates a hypercoagulable state that may partially offset bleeding tendencies in women with hemophilia A, but specialized management is still required 2
• Factor VIII levels may increase during pregnancy in carriers of hemophilia A, providing some protection against bleeding complications 3
• Despite this physiological increase, women with severe hemophilia A may still require factor replacement therapy during pregnancy and delivery 4

Antepartum Management

• Monitor factor VIII levels throughout pregnancy, as levels typically increase during pregnancy but may remain insufficient in severe cases 3
• For pregnant women with hemophilia A requiring anticoagulation:
  • LMWH is preferred over vitamin K antagonists during all trimesters of pregnancy 1
  • Avoid oral direct thrombin inhibitors (e.g., dabigatran) and anti-Xa inhibitors (e.g., rivaroxaban, apixaban) during pregnancy 1
• For women with severe hemophilia A, consider prophylactic recombinant factor VIII concentrate to maintain adequate factor levels 4

Intrapartum Management

• Discontinue LMWH at least 24 hours prior to planned delivery or expected time of neuraxial anesthesia 1
• Avoid invasive intrapartum monitoring techniques and instrumental deliveries to reduce the risk of bleeding complications 3
• Consider bolus recombinant factor VIII concentrate immediately before delivery to ensure adequate hemostasis 4
• Regional anesthesia may be performed with appropriate factor replacement and close monitoring 3

Postpartum Management

• Continue anticoagulants for at least 6 weeks postpartum if anticoagulation is indicated 1
• Monitor closely for postpartum hemorrhage, which occurs at higher rates in women with hemophilia A (19% primary PPH reported in one study) 3
• For women who develop acquired hemophilia A postpartum (a rare complication):
  • Be vigilant for unusual presentations such as deep vein thrombosis combined with bleeding 5
  • Diagnose with prolonged aPTT, low factor VIII levels, and Bethesda assay to detect inhibitors 6
  • Treat with immunosuppressive therapy if inhibitors are detected 5

Common Pitfalls and Caveats

• Failure to recognize the increased risk of both bleeding and thrombosis in pregnant women with hemophilia A 5
• Inadequate factor replacement before invasive procedures or delivery, leading to excessive bleeding 3
• Inappropriate use of anticoagulants without factor replacement, potentially exacerbating bleeding risk 5
• Lack of multidisciplinary approach involving hematology, obstetrics, and anesthesiology 3

Special Considerations

• For carriers of hemophilia A, prenatal testing should be considered to determine fetal status 3
• Cesarean section may be preferred for fetuses known or suspected to have hemophilia to reduce the risk of intracranial hemorrhage 3
• Breastfeeding is compatible with the use of warfarin, acenocoumarol, UFH, and LMWH if anticoagulation is required postpartum 1